Database : MEDLINE
Search on : cochlear and diseases [Words]
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[PMID]: 29287879
[Au] Autor:Niu Z; Yan D; Bressler S; Mei L; Feng Y; Liu X
[Ad] Address:Department of Otolaryngology-Head and Neck Surgery, Xiangya Hospital, Central South University, Changsha 410008, China; Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, FL 33136, USA.
[Ti] Title:A novel splicing mutation in SMPX is linked to nonsyndromic progressive hearing loss.
[So] Source:Int J Pediatr Otorhinolaryngol;104:47-50, 2018 Jan.
[Is] ISSN:1872-8464
[Cp] Country of publication:Ireland
[La] Language:eng
[Ab] Abstract:OBJECTIVE: X-linked nonsyndromic hearing impairment is the rarest form of genetic hearing loss and represents only a minor fraction of all cases. The aim of this study was to investigate the cause of X-linked nonsyndromic sensorineural hearing loss in a three-generation American family. METHODS: Whole-exome sequencing and co-segregation analysis were used to identify disease-causing genes. RESULTS: In this study, we described in detail the clinical characteristics of the family and identified a novel frameshift mutation creating a premature stop codon (c.133-1 G > A, p.(Gly45fs*36)) of SMPX. The loss-of-function mutation was co-segregated with the progressive hearing loss phenotype and was absent in 200 normal controls. CONCLUSIONS: We report the first SMPX (DFNX4) mutation in a North American family. Our findings contribute to the existing genotypic and phenotypic spectrum of SMPX associated hearing loss. Furthermore, our data suggest that exome sequencing is promising in the genetic diagnosis of hearing loss.
[Mh] MeSH terms primary: Deafness/genetics
Genetic Diseases, X-Linked/genetics
Hearing Loss, Sensorineural/genetics
Muscle Proteins/genetics
[Mh] MeSH terms secundary: Adolescent
Adult
Aged
Child
Codon, Nonsense
Female
Frameshift Mutation
Genotype
Humans
Male
Middle Aged
Pedigree
RNA Splicing
Young Adult
[Pt] Publication type:JOURNAL ARTICLE
[Nm] Name of substance:0 (Codon, Nonsense); 0 (Muscle Proteins); 0 (SMPX protein, human)
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[Js] Journal subset:IM
[Da] Date of entry for processing:171231
[St] Status:MEDLINE

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[PMID]: 29236904
[Au] Autor:Valente JSP; Corona AP
[Ad] Address:Programa de Pós-graduação Processos Interativos dos Órgãos e Sistemas, Universidade Federal da Bahia - UFBA - Salvador (BA), Brasil.
[Ti] Title:Alterações retrococleares na esclerose sistêmica: relato de casos. Retrocochlear impairments in systemic sclerosis: a case report study.
[So] Source:Codas;29(6):e20160238, 2017 Dec 07.
[Is] ISSN:2317-1782
[Cp] Country of publication:Brazil
[La] Language:por; eng
[Ab] Abstract:PURPOSE: To report three cases of patients with Systemic Sclerosis (SSc) and retrocochlear impairments. METHODS: This is a case report of three individuals with SSc and retrocochlear impairments assisted at a rheumatology outpatient clinic. All individuals underwent Brainstem Auditory Evoked Potential (BAEP) and, when necessary, audiometry. RESULTS: All three individuals presented sensorineural hearing loss. Although no retrocochlear impairment was identified in the basic audiologic evaluation, the BAEP results were altered. CONCLUSION: Retrocochlear impairments were present in the individuals under study, both in the absolute latencies and interpeak interval, thereby demanding the attention of rheumatologists and speech-language pathologists to such changes during the monitoring of SSc patients. The results also show a need for epidemiological studies on the theme.
[Mh] MeSH terms primary: Hearing Loss, Sensorineural/etiology
Scleroderma, Systemic/complications
[Mh] MeSH terms secundary: Aged
Audiometry, Evoked Response
Auditory Threshold
Evoked Potentials, Auditory, Brain Stem
Female
Humans
Male
Middle Aged
Retrocochlear Diseases/etiology
[Pt] Publication type:CASE REPORTS
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[Js] Journal subset:IM
[Da] Date of entry for processing:171214
[St] Status:MEDLINE

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[PMID]: 29513262
[Au] Autor:Erhardt J; Fuhrer E; Gruschke OG; Leupold J; Wapler MC; Hennig J; Stieglitz T; Korvink JG
[Ad] Address:University of Freiburg, Georges-Köhler-Allee 102, Freiburg, 79110, GERMANY.
[Ti] Title:Should patients with brain implants undergo MRI?
[So] Source:J Neural Eng;, 2018 Mar 07.
[Is] ISSN:1741-2552
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Patients suffering from neuronal degenerative diseases are increasingly being equipped with neural implants to treat symptoms or restore functions and increase their quality of life. Magnetic resonance imaging (MRI) would be the modality of choice for diagnosis and compulsory post-operative monitoring of such patients. However, interactions between the MR environment and implants pose severe health risks to the patient. Nevertheless, neural implant recipients regularly underwent MRI examinations, and adverse events were reported rarely. This should not imply that the procedures are safe. More than 300.000 cochlear implant recipients are excluded from MRI unless the indication outweighs excruciating pain. For 75.000 DBS recipients quite the opposite holds: MRI is considered essential part of the implantation procedure and some medical centres deliberately exceed safety regulations which they referred to as crucially impractical. MRI related permanent neurological dysfunctions in DBS recipients have occurred in the past when manufacturer recommendations were exceeded. Within the last decades extensive effort has been invested to identify, characterise, and quantify the occurring interactions. Today we are far from a satisfying solution to achieve a safe and beneficial MR procedure for all implant recipients. To contribute, we intend to raise awareness of a growing concern and want to summon the community to stop absurdities and instead improve the situation for the increasing number of patients. Therefore, we review implant safety in the MRI literature from an engineering point of view, with a focus on cochlear and DBS implants as success stories in clinical practice. We briefly explain fundamental phenomena which can lead to patient harm, and point out breakthroughs and errors made. We end with conclusions and strategies to avoid future implants from being contraindicated to MR examinations. We believe that implant recipients should enter MRI, but before doing so, we should make sure that the procedure is reasonable.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180307
[Lr] Last revision date:180307
[St] Status:Publisher
[do] DOI:10.1088/1741-2552/aab4e4

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[PMID]: 27779564
[Au] Autor:Smeds H; Wales J; Asp F; Löfkvist U; Falahat B; Anderlid BM; Anmyr L; Karltorp E
[Ad] Address:*Department of Clinical Science, Intervention and Technology, Karolinska Institutet †Department of Otolaryngology, Karolinska University Hospital, Stockholm ‡Department of Signals and Systems, Chalmers University of Technology, Gothenburg, Sweden §Department of Special Needs Education, University of Oslo, Oslo, Norway ||Department of Radiology, Karolinska University Hospital ¶Department of Molecular Medicine and Surgery, Karolinska Institutet **Department of Clinical Genetics ††Department of Social Work in Health, Karolinska University Hospital, Stockholm, Sweden.
[Ti] Title:X-linked Malformation and Cochlear Implantation.
[So] Source:Otol Neurotol;38(1):38-46, 2017 01.
[Is] ISSN:1537-4505
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:OBJECTIVE: To evaluate if cochlear implantation is safe and constitutes an option for hearing rehabilitation of children with x-linked inner ear malformation. STUDY DESIGN: Retrospective patient review in combination with a multidisciplinary follow-up. SETTING: Tertiary referral hospital and cochlear implant program. PATIENTS: Ten children with severe-profound mixed hearing loss and radiological findings consistent with Incomplete Partition type 3 cochlear malformation received cochlear implants during the years 2007 to 2015. Nine of the children had a mutation affecting the gene POU3F4 on Xq21. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Surgical events, intraoperative measures and electrical stimulation levels, hearing and spoken language abilities. RESULTS: In all, 15 cochlear implantations were performed. In three cases the electrode was found to be in the internal auditory canal on intraoperative x-ray and repositioned successfully. One child had a postoperative rhinorrhea confirmed to be cerebrospinal fluid but this resolved on conservative treatment. No severe complications occurred. Postoperative electrical stimulation levels were higher in 9 of 10 children, as compared with typically reported average levels in patients with a normal cochlea. Eight patients developed spoken language to various degrees while two were still at precommunication level. However, speech recognition scores were lower than average pediatric cases. CONCLUSION: Cochlear implantation is a safe procedure for children with severe-profound mixed hearing loss related to POU3F4 mutation inner ear malformation. The children develop hearing and spoken language but outcome is below average for pediatric CI recipients.
[Mh] MeSH terms primary: Cochlear Implantation
Hearing Loss, Sensorineural/congenital
Hearing Loss, Sensorineural/surgery
POU Domain Factors/genetics
[Mh] MeSH terms secundary: Child
Child, Preschool
Chromosomes, Human, X
Cochlea/surgery
Cochlear Implantation/adverse effects
Cochlear Implantation/methods
Cochlear Implants
Ear, Inner/abnormalities
Ear, Inner/surgery
Female
Genetic Diseases, X-Linked
Hearing Loss, Sensorineural/genetics
Hearing Tests
Humans
Male
Mutation
Postoperative Complications/epidemiology
Retrospective Studies
Treatment Outcome
[Pt] Publication type:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Name of substance:0 (POU Domain Factors); 0 (POU3F4 protein, human)
[Em] Entry month:1708
[Cu] Class update date: 180307
[Lr] Last revision date:180307
[Js] Journal subset:IM
[Da] Date of entry for processing:161026
[St] Status:MEDLINE

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[PMID]: 29256428
[Au] Autor:Morell M; Lehnert K; IJsseldijk LL; Raverty SA; Wohlsein P; Gröne A; André M; Siebert U; Shadwick RE
[Ad] Address:Zoology Department, University of British Columbia, Vancouver, British Columbia V6T1Z4, Canada.
[Ti] Title:Parasites in the inner ear of harbour porpoise: cases from the North and Baltic Seas.
[So] Source:Dis Aquat Organ;127(1):57-63, 2017 Dec 19.
[Is] ISSN:0177-5103
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract:Peribullar sinuses of harbour porpoises Phocoena phocoena are parasitized with high prevalence by the nematode Stenurus minor. The effect of S. minor on the hearing ability of this species is still undetermined. Here, we review the occurrence of S. minor in the inner ear of harbour porpoises recovered from strandings in the North and Baltic Seas. In particular, we present the results from ears collected in German and Danish waters from 2002 to 2016 and from Dutch waters from 2010 to 2016. While the prevalence of S. minor in pterygoid and peribullar sinuses and tympanic cavity was high in harbour porpoises (66.67% in our cases), its prevalence in the cochlea was rare. Only 1 case out of 129 analysed by either histology, electron microscopy or immunofluorescence showed the presence of a nematode parasite morphologically consistent with S. minor at the most basal portion of the right cochlea. This individual also had severe haemorrhage along the right cochlear spiral, which was likely caused by ectopic S. minor migration. Although this animal might have had impaired hearing in the right ear, it was otherwise in good body condition with evidence of recent feeding. These findings highlight the need to study the effect of parasites on hearing, and other pathological changes that might impair appropriate processing of acoustic information.
[Mh] MeSH terms primary: Labyrinth Diseases/veterinary
Nematode Infections/veterinary
Phocoena/parasitology
[Mh] MeSH terms secundary: Animals
Ear, Inner/parasitology
Ear, Inner/ultrastructure
Labyrinth Diseases/epidemiology
Labyrinth Diseases/parasitology
Nematoda/classification
Nematoda/isolation & purification
Nematode Infections/epidemiology
Nematode Infections/parasitology
North Sea/epidemiology
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180305
[Lr] Last revision date:180305
[Js] Journal subset:IM
[Da] Date of entry for processing:171220
[St] Status:MEDLINE
[do] DOI:10.3354/dao03178

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[PMID]: 28462501
[Au] Autor:Musazzi UM; Franzé S; Cilurzo F
[Ad] Address:Department of Pharmaceutical Sciences, Università degli Studi di Milano, Via G. Colombo, 71, 20133, Milan, Italy. umberto.musazzi@unimi.it.
[Ti] Title:Innovative pharmaceutical approaches for the management of inner ear disorders.
[So] Source:Drug Deliv Transl Res;8(2):436-449, 2018 Apr.
[Is] ISSN:2190-3948
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:The sense of hearing is essential for permitting human beings to interact with the environment, and its dysfunctions can strongly impact on the quality of life. In this context, the cochlea plays a fundamental role in the transformation of the airborne sound waves into electrical signals, which can be processed by the brain. However, several diseases and external stimuli (e.g., noise, drugs) can damage the sensorineural structures of cochlea, inducing progressive hearing dysfunctions until deafness. In clinical practice, the current pharmacological approaches to treat cochlear diseases are based on the almost exclusive use of systemic steroids. In the last decades, the efficacy of novel therapeutic molecules has been proven, taking advantage from a better comprehension of the pathological mechanisms underlying many cochlear diseases. In addition, the feasibility of intratympanic administration of drugs also permitted to overcome the pharmacokinetic limitations of the systemic drug administration, opening new frontiers in drug delivery to cochlea. Several innovative drug delivery systems, such as in situ gelling systems or nanocarriers, were designed, and their efficacy has been proven in vitro and in vivo in cochlear models. The current review aims to describe the art of state in the cochlear drug delivery, highlighting lights and shadows and discussing the most critical aspects still pending in the field.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1705
[Cu] Class update date: 180305
[Lr] Last revision date:180305
[St] Status:In-Data-Review
[do] DOI:10.1007/s13346-017-0384-5

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[PMID]: 29499347
[Au] Autor:Ding S; Xie S; Chen W; Wen L; Wang J; Yang F; Chen G
[Ad] Address:School of Pharmacy, Guangdong Pharmaceutical University, Guangzhou, China; Guangdong Provincial Key Laboratory of Advanced Drug Delivery, Guangdong Pharmaceutical University, Guangzhou, China; Guangdong Provincial Engineering Center of Topical Precise Drug Delivery System, Guangdong Pharmaceutical U
[Ti] Title:Is oval window transport a royal gate for nanoparticle delivery to vestibule in the inner ear?
[So] Source:Eur J Pharm Sci;, 2018 Feb 27.
[Is] ISSN:1879-0720
[Cp] Country of publication:Netherlands
[La] Language:eng
[Ab] Abstract:Drug delivery to the inner ear by nanomedicine strategies has emerged as an effective therapeutic approach for the management of inner ear diseases including hearing and balance disorders. It is well accepted that substance enters the perilymph from the middle ear through the round window membrane (RWM), but the passage through the oval window (OW) has long been neglected. Up to now, researchers still know little about the pathway via which nanoparticles (NPs) enter the inner ear or how they reach the inner ear following local applications. Herein, we engineered fluorescence traceable chitosan (CS) NPs, investigated the NP distribution within cochlear and vestibular organs, and assessed the availability of RWM and OW pathways to NP transport. Intriguingly, there were high levels of CS NPs in vestibular hair cells, dark cells and supporting cells, but negligible ones in cochlear hair cells and epithelial cells after intratympanic administration. However, the NPs were visualized in two cell models, L929 and HEI-OC1 cell lines, and in the hair cells of cochlear explants after co-incubation in vitro. These combined studies implied that CS NPs might enter the vestibule directly through the OW and then preferentially accumulated in the cells of vestibular organs. Thus, in vivo studies were carried out and clearly revealed that CS NPs entered the inner ear through both the RWM and OW, but the latter played a governing role in delivering NPs to the vestibule with vivid fluorescence signals in the thin bone of the stapes footplate. Overall, these findings firstly suggested that the OW, as a royal gate, afforded a convenient access to facilitate CS NPs transport into inner ear, casting a new light on future clinical applications of NPs in the effective treatment of vestibular disorders by minimizing the risk of hearing loss associated with cochlear hair cell pathology.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180302
[Lr] Last revision date:180302
[St] Status:Publisher

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[PMID]: 29363005
[Au] Autor:Sánchez-Rodríguez C; Cuadrado E; Riestra-Ayora J; Sanz-Fernández R
[Ad] Address:European University of Madrid, Calle del Tajo S/N, Villaviciosa de Odón, 28670, Madrid, Spain. carolina.sanchez2@universidadeuropea.es.
[Ti] Title:Polyphenols protect against age-associated apoptosis in female rat cochleae.
[So] Source:Biogerontology;19(2):159-169, 2018 Apr.
[Is] ISSN:1573-6768
[Cp] Country of publication:Netherlands
[La] Language:eng
[Ab] Abstract:Dietary antioxidants, polyphenols, have been found to be beneficial in protecting against the generation of oxidative stress in various diseases associated with aging. Age-related hearing loss (AHL) is the number one neurodegenerative disorder on our aged population. Sprague-Dawley rats divided into five groups according to their age (3, 6, 12, 18 and 24 months old) and treated with 100 mg/day/kg body weight of polyphenols were used. Then, cochleae were harvested to measure caspase activities (- 3, - 8 and - 9), caspase-3 gene expression, ATP levels, Bax, BcL-2 and p53 levels. 8-OHdG levels (marker of DNA oxidative damage) and annexin-V were also measured in cochleae. Increased levels of caspase-3 and 9 in cochlea were observed with age and this effect was attenuated by polyphenol treatment. In addition, ATP and Bcl-2 levels in older rats were recovered after administration of polyphenols, while Bax and p53 levels protein decreased. Oral supplementation with polyphenols also reduces DNA oxidative damage of cochlear cell. Treatment with polyphenols inhibits the activation of age-related apoptotic signaling by decreasing oxidative stress inside the rat cochlea.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180303
[Lr] Last revision date:180303
[St] Status:In-Data-Review
[do] DOI:10.1007/s10522-018-9747-7

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[PMID]: 29488489
[Au] Autor:Lapchenko AS; Kucherov AG; Ivanets IV; Aslamazova VI; Order RY; Yushkina MA
[Ad] Address:Department of Otorhinolaryngology, N.I. Pirogov Russian National Research Medical University, Moscow, Russia, 117997.
[Ti] Title:Istoriia sozdaniia nauchnykh laboratorii na kafedre otorinolaringologii lechebnogo fakul'teta Rossiiskogo natsional'nogo meditsinskogo universiteta im. N.I. Pirogova. [The history of organization of the research laboratories based at the Department of Otorhinolaryngology of the Faculty of General Medicine].
[So] Source:Vestn Otorinolaringol;83(1):11-17, 2018.
[Is] ISSN:0042-4668
[Cp] Country of publication:Russia (Federation)
[La] Language:rus
[Ab] Abstract:This article was designed to describe the history of the establishment and development of the research divisions based at the Department of Otorhinolaryngology of the Faculty of General Medicine, N.I. Pirogov Russian National Research Medical University, including laser, vestibulogical, and audiological laboratories. The authors present an overview of the main research activities and achievements of the Department with special reference to the management of Meniere's disease, cochlear-vestibular disorders associated with sensorineural hearing loss, injuries to the organs of hearing, and diseases of the central nervous system. Also discussed are the peculiarities of the laser-assisted medical care and the possibilities for the application of therapeutic and surgical lasers for the purposes of the practical otorhinolaryngological work.
[Pt] Publication type:ENGLISH ABSTRACT; JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180228
[Lr] Last revision date:180228
[St] Status:In-Data-Review
[do] DOI:10.17116/otorino201883111-17

  10 / 7746 MEDLINE  
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[PMID]: 29198720
[Au] Autor:Luscan R; Mechaussier S; Paul A; Tian G; Gérard X; Defoort-Dellhemmes S; Loundon N; Audo I; Bonnin S; LeGargasson JF; Dumont J; Goudin N; Garfa-Traoré M; Bras M; Pouliet A; Bessières B; Boddaert N; Sahel JA; Lyonnet S; Kaplan J; Cowan NJ; Rozet JM; Marlin S; Perrault I
[Ad] Address:Laboratory of Embryology and Genetics of Human Malformation, INSERM UMR1163, Institute of Genetic Diseases, Imagine and Paris Descartes University, 75015 Paris, France.
[Ti] Title:Mutations in TUBB4B Cause a Distinctive Sensorineural Disease.
[So] Source:Am J Hum Genet;101(6):1006-1012, 2017 Dec 07.
[Is] ISSN:1537-6605
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Leber congenital amaurosis (LCA) is a neurodegenerative disease of photoreceptor cells that causes blindness within the first year of life. It occasionally occurs in syndromic metabolic diseases and plurisystemic ciliopathies. Using exome sequencing in a multiplex family and three simplex case subjects with an atypical association of LCA with early-onset hearing loss, we identified two heterozygous mutations affecting Arg391 in ß-tubulin 4B isotype-encoding (TUBB4B). Inspection of the atomic structure of the microtubule (MT) protofilament reveals that the ß-tubulin Arg391 residue contributes to a binding pocket that interacts with α-tubulin contained in the longitudinally adjacent αß-heterodimer, consistent with a role in maintaining MT stability. Functional analysis in cultured cells overexpressing FLAG-tagged wild-type or mutant TUBB4B as well as in primary skin-derived fibroblasts showed that the mutant TUBB4B is able to fold, form αß-heterodimers, and co-assemble into the endogenous MT lattice. However, the dynamics of growing MTs were consistently altered, showing that the mutations have a significant dampening impact on normal MT growth. Our findings provide a link between sensorineural disease and anomalies in MT behavior and describe a syndromic LCA unrelated to ciliary dysfunction.
[Mh] MeSH terms primary: Leber Congenital Amaurosis/genetics
Microtubules/genetics
Tubulin/genetics
[Mh] MeSH terms secundary: Adult
Binding Sites/genetics
Cells, Cultured
Child
DNA Mutational Analysis
Female
Humans
Male
Microtubules/metabolism
Middle Aged
Mutation, Missense/genetics
Photoreceptor Cells/metabolism
Tubulin/metabolism
Whole Exome Sequencing
[Pt] Publication type:JOURNAL ARTICLE
[Nm] Name of substance:0 (Tubulin)
[Em] Entry month:1801
[Cu] Class update date: 180222
[Lr] Last revision date:180222
[Js] Journal subset:IM
[Da] Date of entry for processing:171205
[St] Status:MEDLINE


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